Management of Nutritional Issues Associated with Alternating Hemiplegia of Childhood

1. Management of Nutritional Issues Associated with Alternating Hemiplegia of Childhood Maria Raspolic MS, RD Santa Clara Valley Medical Center

2. Management of Nutritional Issues Associated with Alternating Hemiplegia of Childhood Objectives: • Review most common nutritional issues in AHC • Provide recommendations to minimize above issues • Review ketogenic diet as treatment for seizures

3. Common Nutritional Issues Associated with AHC • Poor growth • Dehydration • Constipation • Decreased bone mineral density

4. Poor Growth • Inadequate nutrient intakes • Decreased muscle tone • Oral motor dysfunction • Limited growth potential • Frequent illness, fatigue, infections

5. Growth Assessment

6. Intervention • Provide food of high nutritional quality Breakfast: oatmeal, eggs, cream of wheat, yogurt, Carnation Instant Breakfast, meat Lunch/Dinner: refried beans/cheese, lentils, tofu, almond/nut butter, avocados, fatty fish, meat, sweet potatoes, fried rice/mex rice Snacks: milk shakes/smoothies, cheese stick

7. Intervention con’t. • Liquid supplements • Concentrate infant formula to 24-30 kca/oz • Pediasure, Boost Kids Essential, NutrenJr • Ensure, Nutren, Boost • Carnation Instant Breakfast • 1.5 and 2 cal/cc formulas

8. Intervention con’t. 3. Supplemental tube feeding • Child not able to gain weight adequately • Excessive time needed to feed • Difficult decision for parents • Goal: improved quality of life • Dramatic improvement in nutritional status

9. Dehydration • Inadequate fluid intakes • Excessive fluid loss • Need for thickened liquids • Result in constipation, decreased appetite • Kidney stones, UTI, thickening of secretions • Monitor number of diapers, UA

10. How much fluid? 100 cc/kg of body wt for the first 10 kg 50 cc/kg for the second 10 kg 20 cc/kg for the additional kgs 44 lbs :2.2 = 22 kg 1000 + 500 + 40 = 1540 cc 1540: 30 = 51 oz

11. Recomendation Provide hi nutritional value liquids: • Milk, soy, rice, almond, coconut • Smoothies, milkshakes • ? juice

12. Constipation Multifactorial cause • Poor intakes of fluids and solids • Low muscle tone/ GI motility • Low activity levels • Low fiber diet

13. Constipation con’t. • Contributes to poor appetite • Abdominal distention/discomfort • Irritability • Adequate fluid intakes and fiber • Hi fiber foods: cereal (5 gr/serving), legumes • Sweet potatoes, fruits/vegetables • Prune or pear juice

14. Constipation con’t. If additional help needed: Milk of magnesia Lactulose Miralax Benefiber

15. Low Bone mineral Density • Limited ambulation • Inadequate intakes of Ca, Phos, Vit D • Anticonvulsant therapy • Limited sun exposure • If untreated may lead to osteoporosis, bone deformities and fractures

16. How Much Calcium is Needed? 1-3 years: 500mg 4-8 years 800 mg 9-18 years 1300 mg

17. Calcium Sources in Food Food sources/ table

18. Calcium Supplements Calcium Carbonate Viactiv, Tumbs, Caltrate Calcium Citrate Citracal Oyster Shell, Bone Meal

19. Vitamin D • Sunshine Vitamin • Sunblock use prevents Vit D production • Anticonvulsant meds (Phenobarb, Dilantin) • Decreased absorption of Calcium • Limited food sources: fish liver oil, fatty fish, egg yolk, mushrooms, milk (fortified)

20. Vitamin D con’t. Recommend to check blood levels yearly Goal: 30-60 mmol/dl Supplement 1000 IU/day 50 000 IU/ week Vit D3 (cholecalciferol) in the skin by sun expo Vit D2 (ergocalciferol) synthesized by plants 15 min sun exposure prevent Vit D deficiency

21. Vitamin D con’t. Borusiak et al, 2012 128 children receiving one AED 24 % hypocalcemia 25% hypophosphatemia 13% low vit D Phenobarb, Depakote, Trileptal, Dilantin

22. Vitamin and Mineral Deficiencies • Common in children with AHC • Complete MVI recommended • Chewable tablet preferred • Liquid/soft gummy vits less minerals • Bugs Bunny, Flinstone’s, Scooby Doo, NanoVites

23. Ketogenic Diet How does it work? • Brain needs glucose from food • 24 hours supply • Breakdown of fat produces ketones • ??? Prevention of seizures

24. Chances of success? • 2-3 months trial • 30% of the children seizure free • 30% significant reduction in seizures, reduction in medication or no medication • Reminder do not respond or find it to hard to continue

25. Side Effects of the diet Dehydration-check urine daily with keto stick Constipation-MOM, Miralax Kidney Stones- UA, trace amount of blood Nutrient deficiency- complete MVI, ck blood levels ZN, Se, Vit D Decreased growth- adjust protein, kcal Hi Cholesterol- replace butter with olive oil, supplement with carnitine

26. Initiation of the diet • Gradual decrease in CHO over one week • 2-3 day hospital admission • Fasting only in the AM • At lunch time full keto meal • Allow fluids to meet hydration need • Spec. gravity and ketones check with every void

27. Initiation of the Diet con’t. • Teach families how to calculate and prepare meals • Keto meal planer • Complexity of meals controlled by parents • Ready to feed Ketogenic formula; Ketocal • RCF for tube feeding

28. Discontinuation of the diet • Reduction of the ratio over couple of months • Most parents find diet easier than anticipated • Ketogenic diet is the most effective available treatment for intractable epilepsy today • Atkins diet

29. Any Questions or Comments?